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Pénfigo

Enno Schmidt1, Michael Kasperkiewicz2, Pascal Joly3

  • 1Department of Dermatology, University of Lübeck, Lübeck, Germany; Lübeck Institute for Experimental Dermatology, University of Lübeck, Lübeck, Germany.

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|September 10, 2019
PubMed
Resumen
Este resumen es generado por máquina.

El pénfigo es una enfermedad autoinmune rara que causa ampollas. El tratamiento con rituximab ofrece altas tasas de remisión y reduce los efectos secundarios de los corticosteroides en casos de moderada a grave.

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Área de la Ciencia:

  • Dermatología
  • Inmunología
  • Enfermedades autoinmunes

Sus antecedentes:

  • El pénfigo es una enfermedad autoinmune grave con ampollas.
  • Los autoanticuerpos patógenos se dirigen a las proteínas de adhesión desmogleína (Dsg) 1 y Dsg3.
  • Esta unión de anticuerpos provoca la separación de los queratinocitos (acantólisis).

Objetivo del estudio:

  • Para resumir la comprensión de la patogénesis y el diagnóstico del pénfigo.
  • Para resaltar las estrategias terapéuticas actuales, incluidos los corticosteroides y el rituximab.
  • Evaluar la eficacia y los beneficios de rituximab en el tratamiento del pénfigo.

Principales métodos:

  • El diagnóstico implica la microscopia de inmunofluorescencia directa de las biopsias y el ELISA sérico para los anticuerpos anti-Dsg.
  • El tratamiento principal incluye corticosteroides.
  • El rituximab (anticuerpo anti-CD20) se utiliza para los casos moderados a severos.

Principales resultados:

  • Rituximab induce una remisión completa después de la terapia en el 90% de los pacientes.
  • El rituximab permite una disminución rápida de los corticosteroides.
  • Esto conduce a efectos significativos de ahorro de corticosteroides y a una reducción de los eventos adversos.

Conclusiones:

  • El rituximab es un tratamiento altamente eficaz para el pénfigo de moderado a severo.
  • Mejora significativamente los resultados del paciente al permitir la reducción de los corticosteroides.
  • El rituximab ofrece un gran beneficio en el tratamiento del pénfigo con menos efectos secundarios.